Characterisation of severe obliterative bronchiolitis in rheumatoid arthritis

¹ Hospices civils de Lyon, Centre Hospitalier Lyon Sud, Service de Pneumologie, Pierre-Bénite
² Hospices civils de Lyon, Hôpital Louis Pradel, Service de pneumologie – Centre de Référence des maladies pulmonaires rares de l'adulte; Université de Lyon, Université Lyon I, UCBL-INRA-ENVL-EPHE, UMR754, IFR128, Lyon
³ AP-HP, Hôpital Tenon, Service de Pneumologie et Réanimation - Centre de Compétence des maladies pulmonaires rares de l'adulte, Paris
⁴ Cliniques Universitaires UCL de Mont-Godinne, Yvoir
⁵ Service de Médecine Interne et Pneumologie, Hôpital de la Cavale Blanche, Brest
⁶ Hospices civils de Lyon, Hôpital Louis Pradel, Service de Cytologie et Anatomie Pathologique, Lyon, France

^* To whom correspondence should be addressed. E-mail: germop{at}univ-lyon1.fr.

	Abstract

The characteristics of patients with rheumatoid arthritis (RA) who develop obliterative bronchiolitis characterised by severe airflow obstruction have been hitherto poorly investigated. We conducted a retrospective study of 25 patients with RA and functional evidence of obliterative bronchiolitis (FEV₁/FVC<50% and/or RV/TLC>140% of predicted). Patients (64±11 years) included 17 never-smokers and 8 ex-smokers (10.5±5.4 pack-years). The diagnosis of RA preceded respiratory symptoms in 88% of cases. dyspnoea on exertion was present in all patients and bronchorrhea in 44%. HRCT findings included: bronchial wall thickening (96%), bronchiectasis (40%), mosaic pattern (40%), centrilobular emphysema (56%), reticular and/or ground glass opacities (32%). Pulmonary function tests showed: FEV₁ 41±12% of predicted, FEV₁/FVC 49±14%, FVC 70±20% of predicted, RV 148±68% of predicted, and RV/TLC 142±34% of predicted. Lung biopsy available in 9 patients demonstrated constrictive, follicular, and mixed bronchiolitis. Patients were followed for a mean of 48.2±49 months. Treatment was poorly effective. Chronic respiratory failure occurred in 40% of patients, and 4 patients died. Obliterative bronchiolitis associated with RA is a severe and underrecognised condition leading to respiratory failure and death in a high proportion of patients.